Diagnosis and classification of von Willebrand disease

2011 ◽  
Vol 22 (7) ◽  
pp. 553-564 ◽  
Author(s):  
Emmanuel J. Favaloro
Keyword(s):  
Von Willebrand Disease ◽  
Diagnosis And Classification ◽  
Von Willebrand

scholarly journals Evaluation of a new semi-automated Hydragel 11 von Willebrand Factor multimers assay kit for routine use

2020 ◽  
Author(s):  
Marika Pikta
Keyword(s):  
Von Willebrand Factor ◽  
Quantitative Estimation ◽  
Von Willebrand Disease ◽  
Diagnostic Process ◽  
Routine Practice ◽  
Significant Difference ◽  
Diagnosis And Classification ◽  
Von Willebrand ◽  
Willebrand Factor

Accurate diagnosis and classification of von Willebrand disease are essential for optimal management.  The von Willebrand factor multimers analysis is in the phenotypic classification, especially in discrepant cases, an integral part of the diagnostic process. The aim of this study was to evaluate the performance of a new Hydragel 11VWF multimer assay (H11VW). Results: Comparison study did not reveal any significant difference between H5VW and H11VW.  The assessment of within-subject results, using H5VW and H11VW, demonstrates the reproducibility of the method in both healthy and VWD patients’ samples collected over time, with identical multimeric pattern on densitometric curves.Conclusion: This assay demonstrated acceptable performance, produced within-day results and can be used in routine practice for the visual investigation of gel and quantitative estimation of VWF multimer fractions.

A molecular approach to the classification of von Willebrand disease

2001 ◽  
Vol 14 (2) ◽  
pp. 281-298 ◽  
Author(s):  
Reinhard Schneppenheim ◽  
Ulrich Budde ◽  
Zaverio M. Ruggeri
Keyword(s):  
Von Willebrand Disease ◽  
Molecular Approach ◽  
Von Willebrand

Classification of von Willebrand disease: emerging issues

Haemophilia ◽  
1999 ◽  
Vol 5 (s2) ◽  
pp. 46-49 ◽  
Author(s):  
L. Holmberg ◽  
S. Lethagen
Keyword(s):  
Von Willebrand Disease ◽  
Emerging Issues ◽  
Von Willebrand

A Revised Classification of von Willebrand Disease

1994 ◽  
Vol 71 (04) ◽  
pp. 520-525 ◽  
Author(s):  
J Evan Sadler
Keyword(s):  
Molecular Weight ◽  
High Molecular Weight ◽  
Von Willebrand Disease ◽  
Platelet Glycoprotein ◽  
Dependent Function ◽  
Severe Deficiency ◽  
Partial Deficiency ◽  
Deficiency Type ◽  
Von Willebrand

SummaryA simplified phenotypic classification of von Willebrand disease is proposed that is based on differences in pathophysiology. Quantitative defects arc divided into partial deficiency (type 1) and severe deficiency (type 3). Qualitative defects (type 2) are divided into four subcategories. Type 2A refers to variants with decreased platelet-dependent function associated with the loss of high-molecular weight VWF multimers. Type 2B refers to variants with increased affinity for platelet glycoprotein lb. Type 2M refers to qualitatively abnormal variants with decreased platelet-dependent function not associated with the loss of high-molecular weight multimers. Type 2N refers to variants with decreased affinity for factor VIII. When recognized, mixed phenotypes caused by compound heterozygosity are indicated by separate classification of each allele. Standard amino acid and nucleotide numbering schemes are recommended for the description of mutations.

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